Primary Sjögren syndrome

Definition

Primary Sjögren syndrome is a progressive, systemic inflammatory autoimmune disease affecting primarily the exocrine glands in the absence of other systemic autoimmune diseases. Extraglandular manifestations, which can be divided into two categories: periepithelial organ involvement (i.e., interstitial nephritis, liver involvement), and obstructive bronchitis, is the result of lymphocytic invasion in the epithelia of organs beyond the exocrine glands. Extraepithelial manifestations, such as palpable purpura, glomerulonephritis, and peripheral neuropathy, are produced by an immune complex deposition resulting from the ongoing B-cell hyper-reactivity. The latter manifestations are associated with increased risk for developing lymphoma.

Index Terms